Download Subpulmonary Obstruction Due to Aneurysmal Ventricular Septum

University Heart Journal
Vol. 10, No. 1, January 2014
CASE REPORTS
Subpulmonary Obstruction Due to Aneurysmal Ventricular
Septum in a Patient with Congenitally Corrected
Transposition of the Great Arteries and Dextrocardia
NAVEEN SHEIKH, SAJAL KRISHNA BANERJEE, MD. ZAHID HOSSAIN, MD. TARIQUL ISLAM, TAHMINA KARIM,
ATM IQBAL HASAN, DIPAL KRISHNA ADHIKARY, MD NAZMUL HASAN, MD. HARISUL HOQUE
Department of Cardiology, Bangabandhu Sheikh Mujib Medical University,Shahbag, Dhaka.
Address for Correspondence: Dr Naveen Sheikh,Assistant Professor, Department of Cardiology, Bangabandhu Sheikh Mujib
Medical University,Shahbag, Dhaka. Email: [email protected]
Abstract:
Congenitally corrected Transposition of Great Arteries is usually associated with multiple cardiac defects.
Morphologic left-ventricular outflow (pulmonary) tract obstruction due to aneurysm of the membranous
ventricular septum in patients with corrected transposition and ventricular septal defect is rare, but was reported
in the past. This is even more uncommon in patients with dextrocardia, prompting us to document this case.
Absence of the conus with resultant proximity of the aneurysm to the subpulmonary region and higher
pressures in the left-sided morphologic right ventricle lead to obstruction of outflow tract in corrected
transposition. Echocardiogram with Doppler interrogation and cardiac catheterization with selective
cineangiography are the diagnostic tests of choice. Surgical resection of the aneurysm with patch closure of
ventricular septal defect, avoiding injury to the conduction system, is recommended.
Key Words: Aneurysm, Dextrocardia,Transposition of the Great Arteries.
Introduction:
Congenitally Corrected Transposition of the Great Arteries
is usually associated with multiple cardiac defects. Some
defects are complex and others are simple, such as atrial or
ventricular septal defects. Membranous ventricular septal
defects tend to close spontaneously; such closures are
usually due to plastering down of one of the leaflets of the
tricuspid valve, commonly referred to as formation of
aneurysm of the membranous ventricular septum. Rarely,
the aneurysmal tissue occluding the ventricular septal
defect may prolapse into the outflow tract of the
morphologic left ventricle and cause significant
obstruction to the pulmonary outflow tract, requiring
surgical therapy. We report a case of severe subpulmonary
stenosis due to an aneurysm of the membranous ventricular
septum in a patient with Congenitally Corrected
Transposition of Great Arteries and Dextrocardia.
Case Report:
A three-year-old asymptomatic male child was referred to
us for evaluation of dextrocardia. On cardiovascular
examination, the apical impulse was felt in the right
midclavicular line at the 5th intercostal space with: right
precordial heave, a normal first sound at the right apex, a
single second heart sound at right upper sternal border
and a grade III/IV ejection systolic murmur best heard at
the right upper sternal border. Liver edge was palpable in
the left upper quadrant of the abdomen. There were no
clinical signs of congestive heart failure.
Chest x-ray (Figure 1) revealed dextrocardia, left-sided liver
and left-to-right reversal of bronchi, indicating situs
inversus totalis. The electrocardiogram showed negative
P waves in lead I suggestive of atrial inversion and Q
waves in all chest leads (Figure 2).
Echocardiogram revealed dextrocardia with atrial
situsinversus, atrio- ventricular discordance, D-loop of
Fig.-1: Chest X-ray in posterior-anterior view showing
dextrocardia, reversal of right (MRB) and left (MLB)
main stem bronchi and left sided liver.
University Heart Journal
Vol. 10, No. 1, January 2014
Fig.-2: Electrocardiogram showing negative P waves in lead I suggestive of atrial inversion and Q waves in all chest
leads.
ventricles, and ventriculo-arterial discordance. The
features are consistent with corrected transposition
physiology. There was a moderate-sized perimembranous
ventricular septal defect with left-to-right ventricular
shunting (Figure 3). There was aneurysmal tissue beneath
the pulmonary valve (Figure 4) causing severe sub
pulmonary stenosis with a peak Doppler flow velocity in
excess of 5.0 m/s with a peak instantaneous gradient of
110 mmHg and a mean of 59 mmHg (Figure 5).
Cardiac catheterization and selective cineangiography
confirmed the diagnosis of dextrocardia and atrial
situsinversus. The left-sided morphologic right atrium was
connected to the left-sided morphologic left ventricle
which gave rise to the pulmonary artery (Figure 6A). The
right-sided morphologic left atrium was connected to the
right sided morphologic right ventricle which gave rise to
the aorta (Figure 6B). The aortic valve is anterior (not
shown), superior (Figure 6) and to the right (Figure 6) of
Fig.-3: Two-dimensional (A) and color flow mapping (B) images of apical four-chamber echocardiographic views
obtained from the right chest demonstrating moderate to large ventricular septal defect (VSD). MLV, morphological
left ventricle; MRV, morphological right ventricle.
32
Subpulmonary Obstruction Due to Aneurysmal Ventricular Septum
Naveen Sheikh et al.
Fig.-4: (A) Selected video frame from a long axis two-dimensional echocardiographic view of the morphological left
ventricle (MLV) showing aneurismal tissue (Anu) protruding into the pulmonary outflow tract, causing obstruction. The
Anu is located beneath the pulmonary valve (PV). Dilated main pulmonary artery (MPA) is also seen. (B.) The Anu is also
demonstrated in the subcostal four chamber view. (C) Magnified view of B, again demonstrating the Anu and dilated MPA.
Fig.-5: (A) Selected video frame from a subcostal four chamber view (similar to figure 4B) with color flow mapping
demonstrating turbulent flow (TF) in the pulmonary outflow tract. (B) Continuous wave Doppler recording across
the pulmonary outflow tract shows peak Doppler velocity in excess of 5 m/sec suggesting severe obstruction. See the
text for the calculated gradients. MLV, morphological left ventricle.
pulmonary valve (D-loop). These data confirmed
echocardiographic findings of corrected transposition
physiology. The systolic pressure in the left-sided,
morphologic left (pulmonary) ventricle was at systemic
level, but pulmonary artery pressure was normal. There
was 57 mmHg peak-to-peak systolic pressure gradient
across the left ventricular (pulmonary) out flow tract. Rightsided morphologic right ventricular (systemic) and aortic
pressures were normal. Selective morphologic left
ventricular angiography revealed an aneurysm protruding
into the subpulmonary region causing severe outflow tract
obstruction (Figures 7 & 8). There was a moderate-sized
ventricular septal defect with left-to-right ventricular
shunting (Figure 7 A). It was recommended that the patient
undergo surgical resection of the aneurysmal tissue along
with closure of the ventricular septal defect.
Intraoperative transesophageal echocardiographic
findings are consistent with those of the transthoracic
echocardiographic and angiographic data. Intraoperative
findings were a moderate-sized ventricular septal defect
with tricuspid valve tissue prolapsing through the defect
forming an aneurysm protruding into the left-sided,
morphologic left (pulmonary) ventricular outflow tract. The
patient underwent resection of the aneurysmal tissue and
closure of the ventricular septal defect with a Dacron patch.
He developed complete heart block after the surgery, for
which he received a permanent pacemaker at the same
time. Currently, he is followed in the pediatric cardiology
clinic, and at the last visit eighteen months after surgery,
he was asymptomatic, had no residual ventricular septal
defect but has mild residual pulmonary outflow tract
obstruction with a Doppler peak instantaneous gradient
of 30 mmHg and a mean of 16 mmHg.
33
University Heart Journal
Vol. 10, No. 1, January 2014
Fig.-6: (A) Selected cineangiographic frame from a left-sided ventriculogram in a posterio-anterior view showing a
finely trabeculated, left-sided, morphological left ventricle (MLV) which gives rise to a dilated main pulmonary
artery (MPA). Also note that the inferior vena cava (IVC) is on the left of the spine which is connected the left-sided
right atrium (not shown). (B) Right-sided ventricular angiogram, also in anterio-posterior view, showing a coarsely
trabeculated, right-sided, morphologic right ventricle (MRV) which gives rise to the aorta (AAo). Note that the
descending aorta (DAo) ison the right of the spine. The aortic valve is anterior (not shown),superior and to the right
of pulmonary valve. These data would indicate D-loop of the ventricle, D-transposition of the great arteries in a
subject with dextrocardia, a scenario indicative of corrected transposition physiology.
Fig.-7: Selected cineangiographic frames from morphological left ventricular (MLV) angiogram in 60° left anterior
oblique (A) and lateral (B) views showing pulmonary outflow tract obstruction from aneurismal tissue (thin arrows),
ventricular septal defect (VSD) (thick arrow) and post-stenotic dilation of the main pulmonary artery (MPA). PG,
pigtail catheter in the descending aorta.
34
Subpulmonary Obstruction Due to Aneurysmal Ventricular Septum
Fig.-8: Selected cineangiographic frame from
morphological left ventricular (MLV) angiogram in 60°
left anterior oblique view showing severe narrowing of
the right ventricular outflow tract (double arrows) due
obstruction from aneurismal tissue (not shown) and poststenotic dilation of the main pulmonary artery (MPA).
PG, pigtail catheter in the descending aorta.
Discussion:
Congenital Corrected Transposition of the Great Arteries
was originally described by von Rokitansky in 1875 ¹.
Several case series have been reported since 1950
documenting associated lesions and hemodynamic
abnormalities. The incidence of this lesion is 1 in 33,000
live births which is approximately 0.05% of congenital
cardiac malformations.2,4 The majority are seen with situs
solitus and only 5% are associated with situs inversus,5
similar to our case. The hallmark of the lesion is the so
called “double discordance:” atrio-ventricular and
ventriculo-arterial discordance. Because of this double
discordance, the circulatory physiology is normal;
systemic venous return goes into the lungs and the
pulmonary venous return to the body.6,7 The most
common anatomical arrangement is levocardia with
situssolitus, l-loop of the ventricles and anterior aorta
which is located to the left of the pulmonary artery (S,L,L).
The less common form is Dextrocardia with situsinversus,
d-loop of the ventricles and anterior aorta which is
rightward (I,D,D), as in our case. The most common lesions
are: Ebstein’s malformation of the morphologic tricuspid
valve, ventricular septal defect, morphologic left ventricular
(pulmonary) outflow tract obstruction and complete heart
block.8,10
Naveen Sheikh et al.
Extensive study of the atrioventricular conduction system
in corrected transposition with situs solitus patients by
several investigators11,16 determined that it is abnormally
positioned, coursing in the anterior aspect of the
subpulmonary tissue and along the anterior rim of the
ventricular septal defect. In patients with small or atretic
pulmonary trunk and normal septal alignment, the
conduction system may consist of dual atrioventricular
nodes with sling-like arrangement of conduction tissue.17
The location of the atrioventricular conduction system is
more variable in patients with situsinversus with corrected
transposition physiology; Wilkinson et al18 reported that
the atrioventricular conduction system is usually situated
in the posterior and inferior margin of the ventricular septal
defect (anterior node may be present, but does not connect
to the ventricular myocardium), in contrast to the superior
and anterior location found in corrected transposition of
the great arteries in situssolitus. These observations were
similar to those reported by Dick and associates19 in
intracardiac electrophysiological study during surgery and
by Thiene et al20 in post-mortem hearts. These abnormal
courses of the conduction system are of great surgical
importance, especially in the presence of a ventricular
septal defect or subpulmonary obstruction, since the
conduction system is vulnerable to injury during surgical
repair of the ventricular septal defect, with the potential
for producing iatrogenic heart block, as in our case. Careful
review of the location of the conduction system in a given
situation (levocardia vs. dextrocardia) and, if necessary,
intraoperative electrophysiological definition of the
conduction system19 may be necessary to prevent such a
complication. This lesion is also prone to develop re-entry
tachyarrhythmia and varying degrees of heart block. In
this review we will focus on morphologic left ventricular
(pulmonary) outflow tract obstruction. Issues related to
pulmonary outflow tract obstruction in corrected
transposition have been addressed in both early and more
recent reports.21,27
According to these authors the pulmonary outflow tract
obstruction is due to several causes, and the most common
causes are pulmonary valve stenosis or atresia and
subvalvar pulmonary stenosis related to muscular
malalignment and/or hypertrophy. Other less common
causes are: fibrous tags or accessory valve tissue,28
aneurysm of the membranous system,29,34 tuberculoma
34 and intracardiac blood cyst. 35 Pulmonary outflow
obstruction due to a prolapsing aneurysm of the
membranous ventricular septum is rare, including a detailed
clinical, trans- thoracic and trans-esophageal
echocardiographic, catheterization and angiographic and
35
University Heart Journal
surgical description of this entity in 1996 in a situssolitus
patient by the senior author. Aneurysms of membranous
ventricular septum are commonly seen in association with
perimembranous ventricular septal defects in patients with
normally related great arteries36,38 and constitute one of
the most common mechanisms by which the ventricular
septal defects close spontaneously. Although popularly
called “ventricular septal aneurysm,” it may not be a true
aneurysm, nor derived from the ventricular septum. The
origin of the aneurysm is difficult to ascertain even in
pathologic studies; these studies suggest that this pouch
(aneurysm) is derived either from redundant tricuspid valve
tissue or either from membranous septum itself.38,40 Even
though these closures with aneurysms are beneficial in
many patients with ventricular septal defect, sometimes
the aneurysms can cause obstruction of the pulmonary
outflow tract.
In patients with normally related great arteries, the
aneurysm rarely causes outflow tract obstruction due to
interposition of the conal septum and crista
supraventricularis between the aneurysm and pulmonary
valve. By contrast, in patients with Transposition of the
Great Arteries who have higher right ventricular pressure,
the aneurysm protrudes into the left ventricular outflow
tract and causes pulmonary outflow tact obstruction.41
Similarly, in patients with corrected transposition, in
absence of conal septum and crista supraventricularis in
the morphologic left ventricle the aneurysm is closer to
the pulmonary valve leading to pulmonary outflow tract
obstruction even when the aneurysm is small. According
to the previous reports the age of presentation of this
condition varies from 5 to 54 years, with a median age of 8
years; most patients were male. Our patient is 3-years-old
and is younger than any patient reported so far in the
literature. The presentation may be a cardiac murmur in an
otherwise asymptomatic patient or symptomatic with chest
pain, exertionaldyspnea or easy fatigability. Precordial
heave and a single loud second heart sound are present.
A loud ejection systolic murmur of pulmonary stenosis at
the right upper sternal border and a holosystolic murmur
of ventricular septal defect at the right lower sternal border
may be heard in patients with dextrocardia. If there is
Ebstein’s anomaly, a holosystolic murmur due to
regurgitation of morphologic tricuspid valve is heard at
the apex with radiation to the axilla. Cyanosis and clinical
signs of heart failure are uncommon.
Electrocardiogram shows negative P waves in lead I
suggestive of atrial inversion and abnormal Q waves in
the chest leads. Chest x-ray confirms the dextrocardia,
36
Vol. 10, No. 1, January 2014
left-to-right reversal of the bronchi and left sided liver,
suggestive of situsinversus (Figure 1). Echocardiogram
confirms the diagnosis of atrial inversion, atrio-ventricular
discordance, ventricular inversion, and ventriculo-arterial
discordance and specific relationship of the great
vessels.42,43 Echocardiographic studies are also useful in
demonstrating aneurismal pouch protrusion into the
morphologic left ventricular (pulmonary) outflow tract
(Figure 4) and ventricular septal defect (Figure 3). Doppler
interrogation quantifies the gradient across the obstruction
(Figure 5). Cardiac catheterization is useful as an additional
diagnostic tool to accurately measure the peak- to-peak
systolic pressure gradient across the pulmonary outflow
tract.
Angiography clearly demonstrates the ventricular
morphology (Figure 6), location and size of the aneurysm
(Figure 7) and of the associated lesions. In the current era
cardiac magnetic resonance imaging may be used as an
alternative to angiography. 44 Three-dimensional
echocardiogram45 is another helpful diagnostic tool.
Transesophageal echocardiogram is useful during surgery
to reconfirm the diagnosis and to evaluate the extent of
relief after surgery.46 Treatment of choice is surgical
resection of the obstruction and closure of ventricular
septal defect, although some investigators used valved
conduit to bypass the obstruction. Complete heart block
is a common complication for patients with corrected
transposition leading some investigators47,48 to advocate
intra-operative electrophysiological identification of the
conduction system. deLeval et al49 described a technique
of placing the sutures on the morphologically right side of
the septum without opening the systemic ventricle to close
the ventricular septal defect and reported no significant
arrhythmias related to closure of ventricular septal defect.
Various other surgical strategies, particularly doubleswitch,50,53 have also been described as ways to restore
the morphologic left ventricle to pump against systemic
circulation; some physicians54,55 consider double-switch
preferable to conventional repair.
References:
1.
Von Rokitansky C. Dei Defekte Der Scheidewande Des
Herzens. Vienna: Wilhelm Braumuller, 1875:83-5.
2.
Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart
disease: Prevalence at live birth. The Baltimore—Washington
Infant Study. Am J Epidemiol 1985; 121:31-36.
3.
Fyler DC. Report of the New England Regional Infant Cardiac
Program.Pediatrics 1980; 65(suppl):376-461.
4.
Samanek M, Voriskova M. Congenital heart disease among
815,569 children born between 1980 and their 15-year
Subpulmonary Obstruction Due to Aneurysmal Ventricular Septum
Naveen Sheikh et al.
survival: A prospective Bohemia survival study. PediatrCardiol
1999; 20:411-417. 5. Witham AC. Double outlet right
ventricle: A partial transposition complex. Am Heart J 1957;
53:928-939.
20. Thiene G, Nava A, Rossi L. The conduction system in corrected
transposition with situsinversus.Eur J Cardiol 1977; 6:57-70.
6.
Reddy SC, Chopra PS, Rao PS. Aneurysm of the membranous
ventricular septum resulting in pulmonary outflow tract
obstruction in congenitally corrected transposition of the
great arteries. Am Heart J 1997; 133:112-119.
7.
Wallis GA, Debich-Spicer D, Anderson RH. Congenitally
corrected transposition.Orphanet J Rare Dis 2011; 14:6-22.
8.
Friedberg DZ, Nadas AS.Clinical profile of patients with
congenital corrected transposition of the great arteries.A study
of 60 cases. N Engl J Med 1970; 282:1053-1059.
9.
Allwork SP, Bentall HH, Becker AE, et al. Congenitally
corrected transposition of the great arteries: Morphologic
study of 32 cases. Am J Cardiol 1976; 38:910-923.
10. Anderson KR, Danielson GK, McGoon DW, et al. Ebstein’s
anomaly of the left- sided tricuspid valve: Pathological
anatomy of the valvular malformation. Circulation 1978;
58:87-91.
11. Anderson RH, Arnold R, Wilkinson JL. The conducting system
in congenitally corrected transposition.Lancet 1973; 1:12861288.
12. Anderson RH, Becker AE, Arnold R, Wilkinson JL. The
conducting
tissues
in
congenitally
corrected
transposition.Circulation 1974; 50:911-923.
13. Fischbach P, Law I, Serwer G. Congenitally corrected ltransposition of the great arteries: Abnormalities of
atrioventricular conduction. ProgPediatricCardiol 1999;
10:37-43.
14. Bharati S, McCue CM, Tingelstad JB, et al. Lack of connection
between the atria and the peripheral conduction system in a
case of corrected transposition with congenital atrioventricular
block. Am J Cardiol 1978; 42:147-153.
15. Daliento L, Corrado D, Buja G, et al. Rhythm and conduction
disturbances in isolated, congenitally corrected transposition
of the great arteries. Am J Cardiol 1986; 58:314-318.
16. Becker AE, Anderson RH.The atrioventricular conduction
tissues in congenitally corrected transposition. In: Embryology
and Teratology of the Heart and the Great Arteries. Leiden:
Leiden University Press, 1978: 29-42.
17. Hosseinpour AR, McCarthy KP, Griselli M, Sethia B, Ho SY.
Congenitally corrected transposition: size of the pulmonary
trunk and septalmalalignment. Ann ThoracSurg 2004;
77:2163-2166.
21. Rutledge JM, Nihill MR, Fraser CD, et al. Outcome of 121
patients with congenitally corrected transposition of the great
arteries.PediatrCardiol
2002;
23:137-145.
(Epub
2002 Feb 19).
22. Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term
outcome in congenitally corrected transposition of the great
arteries: A multi-institutional study. J Am CollCardiol 2000;
36:255-261. 23. Marcelletti C, Maloney JD, Ritter DG,
Danielson GK, McGoon DC, Wallace RB. Corrected
transposition and ventricular septal defect.Surgical
experience.Ann Surg. 1980; 191:751-759.
24. Aeba R, Katogi T, Koizumi K, Iino Y, Mori M, Yozu R.
Apico-pulmonary artery conduit repair of congenitally
corrected transposition of the great arteries with ventricular
septal defect and pulmonary outflow tract obstruction: a 10yearfollow-up. Ann ThoracSurg 2003; 76:1383-1387;
discussion 1387-1388.
25. Oliver JM, Gallego P, Gonzalez AE, Sanchez-Recalde A, Brett
M, Polo L, Gutierrez-Larraya F. Comparison of outcomes in
adults with congenitally corrected transposition with
situsinversus versus situssolitus. Am J Cardiol 2012;
110:1687-1691.
26. Yeh T Jr, Connelly MS, Coles JG, Webb GD, McLaughlin PR,
Freedom RM, C e r r i t o P B , W i l l i a m s W G .
Atrioventricular discordance: results of repair in 127 patients.
J ThoracCardiovascSurg 1999; 117:1190-1203.
27. Shin’oka T, Kurosawa H, Imai Y, Aoki M, Ishiyama M,
Sakamoto T, Miyamoto S,Hobo K, Ichihara Y. Outcomes of
definitive surgical repair for congenitally corrected
transposition of the great arteries or double outlet right
ventricle with discordant atrioventricular connections: risk
analyses in 189 patients. J ThoracCardiovascSurg 2007;
133:1318-1328.
28. Levy MJ, Lillehei CW, Elliott LP, Carey LS, Adams P Jr,
Edwards JE. Accessory valvular tissue causing subpulmonary
stenosis in corrected transposition of great vessels. Circulation
1963; 27(4 Pt 1):494-502.
29. Summerall CP, Clowes GH Jr, Boone JA. Aneurysm of
ventricular septum with outflow obstruction of the venous
ventricle in corrected transposition of great vessels. Am Heart
J 1966; 72:525-529.
30. Greene RA, Mesel E, Sissman NJ. The windsock syndrome:
obstructing aneurysm of the interventricular septum associated
with corrected transposition of the great arteries (Abstract).
Circulation 1967; 36(Suppl II):25.
18. Wilkinson JL, Smith A, Lincoln C, Anderson RH.Conducting
tissues in congenitally corrected transposition with
situsinversus.Br Heart J 1978; 40:41-48.
31. Falsetti HL, Andersen MN. Aneurysm of the membranous
ventricular septum producing right ventricular outflow tract
obstruction and left ventricular failure.Chest 1971; 59:
578-580.
19. Dick M 2nd, Van Praagh R, Rudd M, Folkerth T, Castaneda
AR. Electrophysiologic delineation of the specialized
atrioventricular conduction system in two patients with
corrected transposition of the great arteries in situsinversus
(I,D,D). Circulation 1977; 55:896-900.
32. Krongrad E, Ellis K, Steeg CN, Bowman FO Jr, Malm JR,
Gersony WM. Subpulmonary obstruction in congenitally
corrected transposition of the great arteries due to ventricular
membranous septal aneurysms. Circulation 1976;
54:679-683.
37
University Heart Journal
Vol. 10, No. 1, January 2014
33. Ignaszewski AP, Collins-Nakai RL, Kasza LA, Gulamhussein
SS, Penkoske PA, Taylor DA. Aneurysm of the membranous
ventricular septum producing subpulmonic outflow tract
obstruction. Can J Cardiol 1994; 10:67-70.
45. Bartel T, Muller S. Corrected transposition of the great
arteries: dynamic three- dimensional echocardiography and
volumetry. A new diagnostic tool in intensive care
management.Jpn Heart J 1995; 36:819-824.
34. Santos CL, Moraes F, Moraes CR. Obstruction of the right
ventricular outflow tract caused by a tuberculoma in a patient
with ventricular septal defect and aneurysm of the membranous
septum. Cardiol Young 1999; 9:509-511.
46. Caso P, Acione L, Lange A, et al. Diagnostic value of
transesophageal echocardiography in the assessment of
congenitally corrected transposition of the great arteries in
adult patients. Am Heart J 1998; 135:43-50.
35. Bliddal J, Christensen N, Efsen F. Intracardial blood cyst causing
subpulmonary stenosis in congenitally corrected transposition.
Eur J Cardiol 1977; 5:17-27.
47. Kupersmith J, Krongrad E, Gersony WM, Bowman FO Jr.
Electrophysiologic identification of the specialized conduction
system in corrected transposition of the great arteries.
Circulation 1974; 50:795-800.
36. Varghese PJ, Izukawa T, Celermajer J, Simon A, Rowe RD.
Aneurysm of the membranous ventricular septum. A method
of spontaneous closure of small ventricular septal defect. Am
J Cardiol 1969; 24:531-536.
37. Misra KP, Hildner FJ, Cohen LS, Narula OS, Samet P. Aneurysm
of the membranous ventricular septum. A mechanism for
spontaneous closure of ventricular septal defect. N Engl J
Med 1970; 283:58-61.
38. Freedom RM, White RD, Pieroni DR, Varghese PJ, Krovetz
LJ, Rowe RD. The natural history of the so-called aneurysm
of
the
membranous
ventricular
septum
in
childhood.Circulation 1974; 49:375-384.
39. Chesler E, Korns ME, Edwards JE. Anomalies of the tricuspid
valve, including pouches, resembling aneurysms of the
membranous ventricular septum. Am J Cardiol 1968; 21:661668.
40. Tandon R, Edwards JE. Aneurysmlike formations in relation
to membranous ventricular septum.Circulation 1973; 47:10891097.
41. Vidne BA, Subramanian S, Wagner HR. Aneurysm of the
membranous ventricular septum in transposition of the great
arteries. Circulation 1976; 53:157-161.
42. Hagler DJ, Tajik AJ, Seward JB, Edwards WD, Mair DD, Ritter
DG. Atrioventricular and ventriculoarterial discordance
(corrected transposition of the great arteries). Wide-angle
two- dimensional echocardiographic assessment of ventricular
morphology.Mayo ClinProc 1981; 56:591-600.
43. Sharland G, Tingay R, Jones A, et al. Atrioventricular and
ventriculoarterial discordance (congenitally corrected
transposition of the great arteries): Echocardiographic
features, associations, and outcome in 34 fetuses. Heart 2005;
91:1453-1458.
44. Schmidt M, Theissen P, Deutsch HJ, Dederichs B, Franzen D,
Erdmann E, Schicha H. Congenitally correctedtransposition
of the great arteries (L- TGA) with situsinversustotalis in
adulthood: findings with magnetic resonance imaging.
MagnReson Imaging 2000; 18:417-422.
38
48. Waldo AL, Pacifico AD, Bargeron LM, J a m e s T N , K i r k
l i n J W. Electrophysiological delineation of the specialized
A-V conduction system in patients with corrected
transposition of the great vessels and ventricular septal defect.
Circulation 1975; 52:435-441.
49. de Leval MR, Bastos P, Stark J, et al. Surgical technique to
reduce the risks of heart block following closure of ventricular
septal defect in atrioventricular discordance. J
ThoracCardiovascSurg 1979; 78:515-526.
50. Ilbawi MN, DeLeon SY, Backer CL, et al. An alternative
approach to the surgical management of physiologically
corrected transposition with ventricular septal defect and
pulmonary stenosis or atresia.J ThoracCardiovascSurg 1990;
100:410-415.
51. Imai Y. Double-switch operation for congenitally corrected
transposition. Adv Card Surg 1997; 9:65-86.
52. Imai Y, Seo K, Aoki M, Shin’oka T, Hiramatsu K, Ohta A.
Double-Switch operation for congenitally corrected
transposition. SeminThoracCardiovascSurgPediatr Card
SurgAnnu 2001; 4:16-33.
53. Ilbawi MN, Ocampo CB, Allen BS, et al. Intermediate results
of the anatomic repair for congenitally corrected
transposition. Ann ThoracSurg 2002; 73:594-599; discussion
599-600.
54. Langley SM, Winlaw DS, Stumper O, et al. Midterm results
after restoration of the morphologically left ventricle to the
systemic circulation in patients with congenitally corrected
transposition of the great arteries. J ThoracCardiovascSurg
2003; 125:1229-1241.
55. Shin’oka T, Kurosawa H, Imai Y, et al. Outcomes of definitive
surgical repair for congenitally corrected transposition of the
great arteries or double outlet right ventricle with discordant
atrioventricular connections: risk analyses in 189 patients. J
ThoracCardiovascSurg 2007; 133:1318-1328.